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ACR Open Rheumatology Dec 2023This study aimed to identify risk factors associated with the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).
OBJECTIVE
This study aimed to identify risk factors associated with the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).
METHODS
We conducted a systematic literature review of studies focusing on adult patients classified as having SLE-related PAH by searching the electronic databases Embase, Medline, Medline in-progress, Wanfang, China National Knowledge Infrastructure, Ichushi Web, Kmbase, and KoreaMed. Based on the findings, we conducted a Delphi survey to build expert consensus on issues related to screening for PAH in patients with SLE and on the importance and feasibility of measuring the identified factors in clinical practice.
RESULTS
We included 21 eligible studies for data synthesis. Sixteen factors were associated with an increased risk of SLE-PAH: pericardial effusion, serositis, longer duration of SLE, arthritis, acute and subacute cutaneous lupus, scleroderma pattern on nailfold capillaroscopy, diffusion capacity of carbon monoxide in the lungs (DLCO) <70% predicted, interstitial lung disease, thrombocytopenia, and seven serological factors. Six factors were associated with a decreased risk of SLE-PAH: malar/acute rash, hematologic disorder, renal disorder, higher Systemic Lupus Erythematosus Disease Activity Index score, and two serological factors. Among these, there were six risk factors on which the panelists reached strong or general consensus (peak tricuspid regurgitation velocity on echocardiography >2.8 m/s, pericardial effusion, DLCO <70% predicted, scleroderma pattern on nailfold capillaroscopy, brain natriuretic peptide >50 ng/l, and N-terminal pro-brain natriuretic peptide >300 ng/l). The Delphi panel confirmed the need for a screening tool to identify patients with SLE at high risk of developing PAH and provided consensus on the importance and/or practicality of measuring the identified factors.
CONCLUSION
The risk factors we identified could be used in a screening algorithm to identify patients with SLE with a high risk of developing PAH to facilitate early diagnosis, which could improve prognosis and management of these patients.
PubMed: 37794618
DOI: 10.1002/acr2.11611 -
Minerva Medica Jun 2021
Review
Topics: Acute Disease; Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Female; Herpesvirus 4, Human; Humans; Infant; Infectious Mononucleosis; Male; Middle Aged; Serositis; Serous Membrane; Young Adult
PubMed: 31638357
DOI: 10.23736/S0026-4806.19.06047-6 -
BMC Veterinary Research Dec 2023Accurate measurement of disease associated with endemic bacterial agents in pig populations is challenging due to their commensal ecology, the lack of disease-specific...
Detection and disease diagnosis trends (2017-2022) for Streptococcus suis, Glaesserella parasuis, Mycoplasma hyorhinis, Actinobacillus suis and Mycoplasma hyosynoviae at Iowa State University Veterinary Diagnostic Laboratory.
BACKGROUND
Accurate measurement of disease associated with endemic bacterial agents in pig populations is challenging due to their commensal ecology, the lack of disease-specific antemortem diagnostic tests, and the polymicrobial nature of swine diagnostic cases. The main objective of this retrospective study was to estimate temporal patterns of agent detection and disease diagnosis for five endemic bacteria that can cause systemic disease in porcine tissue specimens submitted to the Iowa State University Veterinary Diagnostic Laboratory (ISU VDL) from 2017 to 2022. The study also explored the diagnostic value of specific tissue specimens for disease diagnosis, estimated the frequency of polymicrobial diagnosis, and evaluated the association between phase of pig production and disease diagnosis.
RESULTS
S. suis and G. parasuis bronchopneumonia increased on average 6 and 4.3%, while S. suis endocarditis increased by 23% per year, respectively. M. hyorhinis and A. suis associated serositis increased yearly by 4.2 and 12.8%, respectively. A significant upward trend in M. hyorhinis arthritis cases was also observed. In contrast, M. hyosynoviae arthritis cases decreased by 33% average/year. Investigation into the diagnostic value of tissues showed that lungs were the most frequently submitted sample, However, the use of lung for systemic disease diagnosis requires caution due to the commensal nature of these agents in the respiratory system, compared to systemic sites that diagnosticians typically target. This study also explored associations between phase of production and specific diseases caused by each agent, showcasing the role of S. suis arthritis in suckling pigs, meningitis in early nursery and endocarditis in growing pigs, and the role of G. parasuis, A. suis, M. hyorhinis and M. hyosynoviae disease mainly in post-weaning phases. Finally, this study highlighted the high frequency of co-detection and -disease diagnosis with other infectious etiologies, such as PRRSV and IAV, demonstrating that to minimize the health impact of these endemic bacterial agents it is imperative to establish effective viral control programs.
CONCLUSIONS
Results from this retrospective study demonstrated significant increases in disease diagnosis for S. suis, G. parasuis, M. hyorhinis, and A. suis, and a significant decrease in detection and disease diagnosis of M. hyosynoviae. High frequencies of interactions between these endemic agents and with viral pathogens was also demonstrated. Consequently, improved control programs are needed to mitigate the adverse effect of these endemic bacterial agents on swine health and wellbeing. This includes improving diagnostic procedures, developing more effective vaccine products, fine-tuning antimicrobial approaches, and managing viral co-infections.
Topics: Humans; Swine; Animals; Mycoplasma hyorhinis; Mycoplasma hyosynoviae; Streptococcus suis; Actinobacillus suis; Mycoplasma Infections; Iowa; Retrospective Studies; Universities; Swine Diseases; Arthritis; Endocarditis
PubMed: 38087358
DOI: 10.1186/s12917-023-03807-w -
Cureus May 2024Introduction Systemic lupus erythematosus (SLE) is a complex autoimmune disease with heterogeneous clinical and laboratory features. The incidence increases markedly in...
Introduction Systemic lupus erythematosus (SLE) is a complex autoimmune disease with heterogeneous clinical and laboratory features. The incidence increases markedly in women. The reason for the predominance of the female gender is still under study. The ethnicity could influence the clinical and serological features of SLE. Material and methods This is a retrospective, descriptive, and longitudinal study. We studied 89 patients diagnosed with childhood-onset systemic lupus erythematosus (cSLE) in our center in 2000-2020 for men and 2010-2020 for women. We investigated disease manifestations, ranging from clinical symptoms to renal involvement, at the time of diagnosis and compared them by gender. Results We studied 89 patients, comprising 23 males and 66 females. The mean age for both groups was 12 years. Concerning clinical manifestations, serositis exhibited a higher prevalence among males, while hair loss was more prominent among females. In the paraclinical evaluation, noteworthy differences were observed regarding average hemoglobin levels and the prevalence of positive anti-DNA antibodies. Males demonstrated an average hemoglobin level of 11.47 g/dL, whereas females displayed 9.84 g/dL (p=0.017). The prevalence of anti-DNA antibodies exhibited marked elevation in males, at 88.9%, compared to females' 42.9% (p=0.024). On a contrary note, our male cohort displayed heightened prevalence in using hydroxychloroquine, cyclophosphamide, and mycophenolate. Similarly, renal involvement presented a higher prevalence in males (100% against 83.3%), albeit lacking statistical significance. Nevertheless, significant disparities emerged in the occurrence of granular casts, proteinuria, and the average glomerular filtration rate, with males experiencing greater impact in each instance. Finally, it is noteworthy that the application of mycophenolate and azathioprine was more frequently observed among patients with renal involvement. Conclusion cSLE patients in our inception cohort showed statistical significance in dermatological and vascular manifestations, serositis, granular casts, low kidney glomerular filtration, and high proteinuria, which are predominant in male patients. Immunological features were predominantly positive in ds-DNA antibodies for male patients. Separation by gender for future studies might identify a better treatment strategy.
PubMed: 38854180
DOI: 10.7759/cureus.59851 -
Clinical and Experimental Medicine Sep 2023Despite an essential differential diagnosis for fever of unknown origin (FUO) in young adults, adult-onset Still's disease (AOSD) is infrequently considered and remained...
Despite an essential differential diagnosis for fever of unknown origin (FUO) in young adults, adult-onset Still's disease (AOSD) is infrequently considered and remained underdiagnosed in low-middle-income countries. The present study analyzed the clinical, serological, radiological, and pathological characteristics of AOSD presented as FUO in India. A hospital-based retrospective study of patients aged > 13 years admitted with FUO and later diagnosed with AOSD in Postgraduate Institute of Medical Education and Research, Chandigarh (India), was conducted between January 2014 and December 2020. Petersdorf and Beeson's criteria were used to define FUO. The diagnosis of AOSD was made based on Yamaguchi's criteria. Twenty-seven patients (median age 26 years, 14 females) were enrolled. All presented with intermittent fever with a median duration of 10 weeks. The typical features of AOSD at admission were arthralgia (n = 24), hepatosplenomegaly (n = 21), spiking fever ≥ 39 °C (n = 19), lymphadenopathy (n = 18), typical rash (n = 17), and sore throat (n = 11). Leukocytosis (n = 25) and neutrophilia (n = 19) were frequent. Hyperferritinemia was universal (range, 700-145,003 ng/ml; ≥ 2000, n = 23). At admission, AOSD was suspected in only nine FUO cases, while tuberculosis (n = 16), undifferentiated connective tissue disorder (n = 14), and lymphoproliferative disorder (n = 11) were common diagnostic possibilities. Crispin et al. clinical scale detected AOSD in only 15 (55.5%) FUO patients. Whole-body imaging (n = 27), including fluorodeoxyglucose positron emission tomography (n = 12), demonstrated reticuloendothelial organ-system involvement and serositis. Seventeen (63%) patients had macrophage activation syndrome at the time of AOSD diagnosis. AOSD FUO presents with typical but nonspecific features; thus, early differentiation from common causes (e.g., tuberculosis, lymphoma) is difficult. Macrophage activation syndrome is common in AOSD with FUO presentation.
Topics: Female; Young Adult; Humans; Adult; Fever of Unknown Origin; Retrospective Studies; Still's Disease, Adult-Onset; Macrophage Activation Syndrome; Diagnosis, Differential
PubMed: 36178600
DOI: 10.1007/s10238-022-00903-3 -
European Review For Medical and... Mar 2020Idiopathic recurrent serositis (IRS) is the most frequent serositis encountered in real-life medical sceneries, and its management represents a therapeutic challenge....
OBJECTIVE
Idiopathic recurrent serositis (IRS) is the most frequent serositis encountered in real-life medical sceneries, and its management represents a therapeutic challenge. There are few epidemiologic data related to IRS, though most studies have focused on recurrent pericarditis, revealing that 70% of all forms of pericarditis are idiopathic and caused by innate immunity abnormalities. The aim of this study was to evaluate outcome and recurrence rates of patients with IRS, assessing management modalities used in our Periodic Fever Centre of the Gemelli Hospital, Rome, Italy, in comparison with previous treatments in other centres.
PATIENTS AND METHODS
Retrospectively, we analyzed the medical charts of 57 unselected patients with history of IRS managed during the period 1998-2017.
RESULTS
A strong heterogeneity emerged by evaluating treatments of this cohort. In particular, in our Centre there was a larger use of combined therapies: 14 patients out of 27 (52%) were treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and colchicine, compared to only 2 patients (7.4%) previously treated with combined treatments. We used corticosteroid monotherapy only in 1 case, against 7 from other centres. The mean duration of NSAID treatment in other hospitals was 43.8 days (SD ±27.40) and 191.25 days (SD ±42.23) in our Centre; the mean duration of corticosteroid treatment in other hospitals was 101.5 days (SD ±56.40) and 180.7 days (SD ±84.87) in our Centre. Colchicine was administered in other hospitals for the same duration of NSAIDs, and corticosteroids with an average duration of 111 days (SD ±30); conversely, we administered colchicine for an average duration of 250.12 days (SD ±80.7). Relapses of IRS were reported in 1/3 of cases who had discontinued therapies.
CONCLUSIONS
The overall duration of treatments to manage IRS has a weight in terms of patients' outcome. A reduced duration of therapy with corticosteroids and a longer duration of therapy with NSAIDs determine a longer disease-free interval. A significant discriminating effect in terms of risk of IRS recurrence relies in an earlier combination therapy with colchicine independently from the start with either NSAIDs or corticosteroids. Finally, the evaluation of genes causing autoinflammatory diseases has not revealed any pathogenetic variants in a subcohort of 20/57 patients with IRS.
Topics: Adrenal Cortex Hormones; Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents, Non-Steroidal; Cohort Studies; Colchicine; Female; Humans; Male; Middle Aged; Recurrence; Retrospective Studies; Rome; Serositis; Time Factors; Young Adult
PubMed: 32271453
DOI: 10.26355/eurrev_202003_20703 -
Annals of Palliative Medicine Sep 2021This retrospective study aimed to investigate the usefulness of neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) for organ involvement and...
BACKGROUND
This retrospective study aimed to investigate the usefulness of neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) for organ involvement and disease activity in newly diagnosed juvenile systemic lupus erythematosus (jSLE).
METHODS
A total of 186 jSLE inpatients were included for analysis. All participants' clinical characteristics and laboratory data were obtained from medical records. The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score scale was used to assess disease activity. Mann-Whitney U test and Kruskal-Wallis test were performed for non-parametric variables between the groups. Spearman rank correlation coefficient was used to analyze correlations between variables.
RESULTS
The NLR was significantly higher in participants with serositis as compared those without serositis [2.72 (1.71-5.0) vs. 2.08 (1.42-3.15), P=0.038]. The PLR was significantly higher in participants manifesting symptoms of cutaneous rash [130.0 (92.6-235.0) vs. 112.0 (49.3-169.0), P=0.002], and arthritis [167.0 (106.0-243.0) vs. 106.0 (53.6-176.0), P<0.001], as compared to participants without such involvement. The PLR in participants with hematological involvement was significantly lower than in those without such involvement [111.0 (53.6-191.0) vs. 138.0 (107.0-185.0), P=0.016]. The PLR in participants with positive anti-Smith (anti-Sm) antibody was significantly higher than that in those with negative anti-Sm antibody [140.0 (91.6-228.0) vs. 114.0 (60.9-176.0), P=0.006]. The NLR showed positive correlations with serositis (r=0.153, P=0.037), complement C3 and C4 (r=0.152, P=0.038 and r=0.177, P=0.016, respectively). The PLR showed positive correlations with cutaneous rash (r=0.227, P=0.002), arthritis (r=0.290, P<0.001), anti-Sm antibodies (r=0.20, P=0.006) and erythrocyte sedimentation rate (ESR, r=0.159, P=0.03). Negative correlations were found between PLR and hematological involvement (r=-0.177, P=0.015).
CONCLUSIONS
Both the NLR and PLR had correlations with serological indicators, and may predict organ involvement in jSLE, particularly cutaneous, arthritis, serositis, and haematological involvement.
Topics: Blood Platelets; Humans; Lupus Erythematosus, Systemic; Lymphocytes; Neutrophils; Retrospective Studies
PubMed: 34628866
DOI: 10.21037/apm-21-1995 -
Biology of Sex Differences Dec 2019Systemic lupus erythematosus (SLE) predominantly affects women, but previous studies suggest that men with SLE present a more severe disease phenotype. In this study, we...
OBJECTIVE
Systemic lupus erythematosus (SLE) predominantly affects women, but previous studies suggest that men with SLE present a more severe disease phenotype. In this study, we investigated a large and well-characterized patient group with the aim of identifying sex differences in disease manifestations, with a special focus on renal involvement.
METHODS
We studied a Swedish multi-center SLE cohort including 1226 patients (1060 women and 166 men) with a mean follow-up time of 15.8 ± 13.4 years. Demographic data, disease manifestations including ACR criteria, serology and renal histopathology were investigated. Renal outcome and mortality were analyzed in subcohorts.
RESULTS
Female SLE patients presented more often with malar rash (p < 0.0001), photosensitivity (p < 0.0001), oral ulcers (p = 0.01), and arthritis (p = 0.007). Male patients on the other hand presented more often with serositis (p = 0.0003), renal disorder (p < 0.0001), and immunologic disorder (p = 0.04) by the ACR definitions. With regard to renal involvement, women were diagnosed with nephritis at an earlier age (p = 0.006), while men with SLE had an overall higher risk for progression into end-stage renal disease (ESRD) with a hazard ratio (HR) of 5.1 (95% CI, 2.1-12.5). The mortality rate among men with SLE and nephritis compared with women was HR 1.7 (95% CI, 0.8-3.8).
CONCLUSION
SLE shows significant sex-specific features, whereby men are affected by a more severe disease with regard to both renal and extra-renal manifestations. Additionally, men are at a higher risk of developing ESRD which may require an increased awareness and monitoring in clinical practice.
Topics: Adult; Disease Progression; Female; Humans; Kidney Diseases; Lupus Erythematosus, Systemic; Male; Middle Aged; Pericarditis; Pleurisy; Serositis; Severity of Illness Index; Sex Characteristics; Sweden; Young Adult
PubMed: 31843005
DOI: 10.1186/s13293-019-0274-2 -
Medicine Dec 2023To investigate the risk factors for the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE). (Meta-Analysis)
Meta-Analysis
BACKGROUND
To investigate the risk factors for the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).
METHODS
The literature related to risk factors for the development of PAH in SLE patients was searched by the computer on China national knowledge infrastructure (CNKI), PubMed, and Embase, and the literature search was limited to the period of library construction to October 2022. Two researchers independently performed literature screening and literature information extracting, including first author, publication time, case collection time, sample size, and study factors, and used the Newcastle-Ottawa Scale (NOS) to evaluate the quality of the literature. The relationship between each clinical manifestation and laboratory index and the occurrence of PAH in SLE patients was evaluated based on the ratio (OR value) and its 95% CI.
RESULTS
A total of 24 publications were included, including 23 case-control studies and 1 cohort study with NOS ≥ 6, and the overall quality of the literature was high. The risk of PAH was higher in SLE patients who developed Raynaud phenomenon than in those who did not [OR = 2.39, 95% CI (1.91, 2.99), P < .05]; the risk of PAH was higher in SLE patients who were positive for anti-RNP antibodies than in those who were negative for anti-RNP antibodies [OR = 1.77, 95% CI (1.17, 3.2.65), P < .05]; the risk of PAH was higher in SLE patients with interstitial lung lesions than in those without combined interstitial lung lesions [OR = 3.28, 95% CI (2.37, 4.53), P < .05]; the risk of PAH was higher in SLE patients with combined serositis than in those without serositis [OR = 2.28, 95% CI (1.83, 2.84), P < .05]. The risk of PAH was higher in SLE patients with combined pericardial effusion than in those without pericardial effusion [OR = 2.97, 95% CI (2.37, 3.72), P < .05]; the risk of PAH was higher in SLE patients with combined vasculitis than in those without vasculitis [OR = 1.50, 95% CI (1.08, 2.07), P < .05]; rheumatoid factor-positive SLE patients had a higher risk of PAH than those with rheumatoid factor-negative [OR = 1.66, 95% CI (1.24, 2.24), P < .05].
CONCLUSION
Raynaud phenomenon, vasculitis, anti-RNP antibodies, serositis, interstitial lung lesions, rheumatoid factor, and pericardial effusion are risk factors for the development of PAH in patients with SLE.
Topics: Humans; Pulmonary Arterial Hypertension; Cohort Studies; Hypertension, Pulmonary; Serositis; Pericardial Effusion; Rheumatoid Factor; Lupus Erythematosus, Systemic; Familial Primary Pulmonary Hypertension; Risk Factors; Raynaud Disease; Vasculitis
PubMed: 38134088
DOI: 10.1097/MD.0000000000036654 -
Frontiers in Immunology 2021This study aimed to describe the main characteristics of Chinese patients with cryoglobulinemia, especially the characteristics of patients with different causes of...
This study aimed to describe the main characteristics of Chinese patients with cryoglobulinemia, especially the characteristics of patients with different causes of cryoglobulinemia. Eighty inpatients diagnosed with cryoglobulinemia from different wards in Peking Union Medical College Hospital were included in this study. Demographic, clinical, biological, and renal pathological data were collected. We analyzed the characteristics of 61 patients with different causes of cryoglobulinemia. Most patients (36/80, 45%) were diagnosed between 40 and 60 years of age. The male: female ratio was 1:1.5. Mixed (II + III) cryoglobulinemia accounted for the majority (43.8%) of cases. Renal involvement (87.5%), cutaneous involvement (57.5%), and fever (27.5%) were the most common clinical manifestations, while other manifestations included serositis and pulmonary and gastrointestinal involvement. The most common renal histopathological pattern was membranoproliferative glomerulonephritis (25/42, 59.5%). The secondary causes of cryoglobulinemia included infectious diseases (26/61, 32.5%), such as hepatitis B virus (HBV) and hepatitis C virus (HCV) infections, and connective tissue diseases (22/61, 27.5%), such as lupus and hematologic tumors (13/61, 16.3%). Patients with hematologic tumors were diagnosed at an older age ( = 0.044) and mostly had type I cryoglobulinemia ( < 0.001). No significant difference in clinical or biological manifestations was found among patients with different causes of cryoglobulinemia. This is the largest cohort of Chinese patients with cryoglobulinemia. We found that renal involvement and HBV infection might be more common in Chinese patients with cryoglobulinemia.
Topics: Adult; Aged; Autoimmunity; Biomarkers; Cryoglobulinemia; Disease Management; Disease Susceptibility; Female; Hepatitis B; Humans; Kidney Diseases; Male; Middle Aged; Prognosis; Symptom Assessment; Young Adult
PubMed: 33717064
DOI: 10.3389/fimmu.2021.580271